Int.J.Curr.Microbiol.App.Sci (2015) 4(7): 339-342
ISSN: 2319-7706 Volume 4 Number 7 (2015) pp. 339-342
http://www.ijcmas.com
Case Study
Rapidly growing infiltrating Glomus jugulare tumor: An uncommon
cause of bleeding aural polyp
Nadia Shirazi1* and SS Bist2
Department of Pathology, Himalayan Institute of Medical Sciences, Swami Rama Himalayan
University, Jolly Grant, Dehradun, Uttarakhand, India
Department of ENT, Himalayan Institute of Medical Sciences, Swami Rama Himalayan
University, Jolly Grant, Dehradun, Uttarakhand, India
*Corresponding author
ABSTRACT
Keywords
Glomus
jugulare,
Paraganglioma,
Infiltrating
Glomus jugulare tumors are rare slow growing tumors occurring within the
jugular foramen. Although benign, these tumors are locally destructive
because of their proximity to the petrous bone, lower cranial nerves and
major vascular structures. We present the case of a 61 year old female
presenting with sudden onset hearing loss and bleeding from ear.
Introduction
Case
Glomus Jugulare Tumors (GJT) are rare
vascular tumors included in the group of
paragangliomas. They usually arise in the
region of jugular bulb however can also be
seen in a variety of sites like carotid body,
glomus vagale and glomus tympanium.
These hypervascular tumors are slow
growing and arise within the jugular
foramen of the temporal bone. GJTs have an
annual incidence of 1 case per 1.3 million
population (Forbes et al., 2012). The tumors
tend to be more common on the left side and
the male:female ratio is 1:3 6. They are seen
in the 5th to 6th decade of life and are rare in
people younger than 18 years of age.
Multicentricity is seen particularly in
familial cases (Moffat and Hardy, 1989;
Havekes et al., 2008).
A 61 year old hypertensive female presented
to the ENT OPD with chief complains of ear
fullness, otorrhea, hemorrhage and a left
aural mass. There was no history of pain,
facial palsy and hoarseness of voice or
dysphagia. Otoscopic examination revealed
a pulsatile reddish mass which appeared to
be originating from the anterior part of
auditory canal (Figure 1). CT scan showed
an infiltrating hypervascularised tumor
which was extending into the middle ear as
well as inferiorly into the infratemporal
fossa. Bony margins were eroded. A
differential
diagnosis
of
jugular
paraganglioma or schwannoma were
rendered radiologically. Patient was taken
up for surgical excision.
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Int.J.Curr.Microbiol.App.Sci (2015) 4(7): 339-342
Conservative
jugulopetrosectomy
was
performed and mass was sent for
histopathological examination (HPE).
are the jugular bulb including internal
auditory canal, the posterior semicircular
canal, the middle ear, medial external
auditory canal, facial nerve (posterolaterally) and the internal carotid artery. On
the basis of extension of tumor into the
adjacent structures, Fisch classification of
glomus tumors is widely used. The
classification is detailed as follows:
On gross examination a reddish brown soft
to firm tissue piece measuring 1.2 x 1 x 0.5
cm was received. Cut section showed a
homogenous appearance. HPE described a
benign tumor forming a zell- ballen
pattern. Cells had round to mildly
pleomorphic nuclei with granular salt and
pepper type chromatin. Mitotic figures were
sparse and necrosis was not seen. Tumor
was involving the posterior surgical resected
margin indicating an incomplete excision
(Photomicrograph
1).
Immunohistochemistry
showed
S-100
positivity in tumor cells. Diagnosis of
glomus
jugulare/
jugulotympanic
paraganglioma was rendered. There were no
post operative complications and the patient
was sent for radiotherapy considering
incomplete primary excision.
Type A: limited to middle ear cleft (glomus
tympanicus)
Type B: limited to tympanomastoid area
with no infralabyrinthine compartment
Type C: Tumor involving infralabyrynthine
compartment of the temporal bone and
extending into the petrous apex
Type C1: Tumor with limited involvement
of the vertical portion of the carotid canal
Type C2-Tumor invading the vertical
portion of the carotid canal
Type C3:-Tumor invasion of horizontal
portion of carotid canal
Type D1- Tumor with intracranial
extension<2cm in diameter
Type
D2-Tumor
with
intracranial
extension>2cm in diameter
Discussion
The GJT are small collections of
paraganglionic tissue which are derived
from embryonic neuroepithelium in close
association with the autonomic nervous
system and are found in the region of jugular
bulb. Based on the presence of
catecholamines
and
neuropeptides,
paraganglia are included in the amine
precursor uptake and decarboxylase (APUD)
system, which has been referred to as diffuse
neuroendocrine system (DNES). The first
description of glomus tumors as hyperplastic
glomus bodies was reported by Masson in
1924. These are rare tumors with benign
characteristics and a slow growth rate of
1mm per year (Jansen et al., 2000). The
main blood supply is via the ascending
pharyngeal artery from the external carotid
artery and branches from the petrous portion
of the internal carotid artery. The structures
lying in close proximity to glomus jugulare
Classifying the tumor according to the
degree of structures infiltrated closely
relates to the mortality and morbidity
associated with the tumor. Although no
known risk factors have been recognized in
GJT, a pathogenetic mutation in the gene for
succinate dehydrogenase enzyme has been
implicated in causing the disease (Pigny et
al., 2008).
Surgery
(complete
resection
with
conservation of cranial nerves) is the
treatment of choice however other
modalities of treatment are embolization,
radiation, gamma knife radiosurgery,
intratumoral injection of cyanoacrylate glue.
Medical therapy may be indicated in tumors
secreting catecholamines ( blockers,
blockers) (Sharma et al., 2008).
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Int.J.Curr.Microbiol.App.Sci (2015) 4(7): 339-342
Figure.1 Otoendoscopy showing a red vascular mass completely filling
the Left external auditory canal
Photomicrograph.1 H&E: 200X: Zell ballen arrangement of glomus jugulare tumor
A study by Prabhu et al. (2004) showed that
even complex glomus tumors can be
managed surgically. Mortality rate is 6.2%
among patients treated with radiation and
2.5% among those treated surgically. The
overall mortality is 8.7% (Rosenwasser et
al., 1945).
significant diagnostic and management
challenge because of the location and extent
of involvement
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Conclusion
Though the tumors are benign, they pose a
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Int.J.Curr.Microbiol.App.Sci (2015) 4(7): 339-342
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